T50 Calciprotein Crystallization and the Decreased Role of Fetuin-A in Type 2 Diabetes.

Aim: Patients with type 2 diabetes mellitus (T2D) are prone to develop vascular calcification. Fetuin-A protects against vascular calcification but it increases insulin resistance. T50 calciprotein crystallization (also called serum calcification propensity) is a novel marker of calcification stress.

Definition of hyperfiltration taking into account age-related decline in renal function in kidney donor candidates with obesity and glucose tolerance disorder.

The definition of hyperfiltration, the main pathogenesis in renal impairment in obesity and diabetes mellitus, is uncertain. Glomerular filtration rate (GFR) declines physiologically with aging, and there is inaccuracy in GFR in obesity due to body surface area (BSA) correction. Here, we defined hyperfiltration using GFR without BSA correction, but with inclusion of aging, and investigated hyperfiltration using this definition and absolute GFR > 125 mL/min.

The Surprise Question in Hemodialysis, Frailty, Nutrition, Patient-reported Quality of Life, and All-Cause Mortality: The Osaka Dialysis Complication Study (ODCS).

Rationale & Objective: A response "no" (SQ-No) to the surprise question (SQ) of whether a clinician would be surprised if a dialysis patient died in the next 6 months is associated with a higher risk of all-cause death. It is uncertain what domains are intuitively assessed with the SQ. We hypothesized that the SQ would assess the patient's frailty, malnutrition, or patient-perceived health-related quality of life in a cohort of patients on maintenance hemodialysis.

Association Between Plasma Aldosterone Concentration and Intraglomerular Hemodynamics in Primary Aldosteronism.

Background: In primary aldosteronism (PA), aldosterone could affect glomerular hemodynamics by elevating renal vascular resistance and glomerular capillary pressure. However, the relationship between plasma aldosterone concentrations (PAC) and glomerular hemodynamics including efferent arteriolar resistance (Re), and afferent arteriolar resistance (Ra) in humans is still unclear. The aim of this study was to investigate the relationships of PAC with intraglomerular hemodynamic parameters in patients with PA.

Protocol for the nationwide registry of patients with polycystic kidney disease: japanese national registry of PKD (JRP).

Background: Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD) are major genetic polycystic kidney diseases that can progress to end-stage kidney disease (ESKD). Longitudinal data on the clinical characteristics associated with clinical outcomes in polycystic kidney disease (PKD), including the development of ESKD and cardiovascular disease (CVD) are lacking in Japan. To address this unmet need the authors are establishing a novel, web-based, Nationwide Cohort Registry Study-the Japanese Registry of PKD (JRP).

Differential associations of fetuin-A and calcification propensity with cardiovascular events and subsequent mortality in patients undergoing hemodialysis.

Background: Fetuin-A inhibits precipitation of calcium-phosphate crystals by forming calciprotein particles (CPP). A novel T50 test, which measures transformation time from primary to secondary CPP, is an index for calcification propensity. Both lower fetuin-A and shorter T50 levels were associated with cardiovascular disease (CVD) risk in patients with chronic kidney disease (CKD).

New-onset Kidney Biopsy-proven Membranous Nephropathy Induced End-stage Kidney Disease in a Living Donor.

Thirteen years after kidney donation, a 70-year-old man was referred to a nephrologist because of proteinuria. The serum creatinine, albumin, and urinary protein levels were 2.39 mg/dL, 3.

Histologic and Clinical Factors Associated with Kidney Outcomes in IgA Vasculitis Nephritis.

Background: Nephritis is a common manifestation of IgA vasculitis and is morphologically indistinguishable from IgA nephropathy. While MEST-C scores are predictive of kidney outcomes in IgA nephropathy, their value in IgA vasculitis nephritis has not been investigated in large multiethnic cohorts.

Methods: Biopsies from 262 children and 99 adults with IgA vasculitis nephritis ( N =361) from 23 centers in North America, Europe, and Asia were independently scored by three pathologists.

Novel Digenic Variants in and Causing X-Linked Alport Syndrome: A Case Report.

Introduction: Alport syndrome (AS) is a hereditary, progressive kidney disease characterized by structural abnormalities and dysfunction of the glomerular basement membrane (GBM). AS is classified as X-linked, autosomal, and digenic. The number of cases of digenic AS has increased, but the genotype-phenotype correlation of patient with digenic AS is still unclear.

Dapagliflozin administration for 1 year promoted kidney enlargement in patient with ADPKD.

To date, there is insufficient evidence regarding use of sodium-glucose cotransporter-2 (SGLT2) inhibitors for patients with autosomal-dominant polycystic kidney disease (ADPKD), as such cases have been excluded from previous clinical trials exploring the kidney protection effects of such medications. Here, findings of an ADPKD patient who received dapagliflozin, a selective SGLT2 inhibitor, for 1 year are presented. A 38-year-old woman with a family history of ADPKD wished for treatment with dapagliflozin.

Short-Term Dapagliflozin Administration in Autosomal Dominant Polycystic Kidney Disease-A Retrospective Single-Arm Case Series Study.

Treatment with sodium-glucose cotransporter-2 (SGLT2) inhibitors may have pleiotropic and beneficial effects in terms of ameliorating of risk factors for the progression of autosomal dominant polycystic kidney disease (ADPKD). However, there is insufficient evidence regarding the use of these drugs in patients with ADPKD, as they were excluded from several clinical trials conducted to explore kidney protection provided by SGLT2 inhibitors. This retrospective single-arm case series study was performed to investigate the effects of dapagliflozin, a selective SGLT2 inhibitor administered at 10 mg/day, on changes in height-adjusted kidney volume (htTKV) and estimated glomerular filtration rate (eGFR) in ADPKD patients.

Public support for patients with intractable diseases in Japan: impact on clinical indicators from nationwide registries in patients with autosomal dominant polycystic kidney disease.

Background: Clinical practice guidelines recommend antihypertensive and tolvaptan therapies for patients with autosomal dominant polycystic kidney disease (ADPKD) in Japan. However, tolvaptan therapy may pose an economic burden. The Japanese Ministry of Health, Labour and Welfare supports patients with intractable diseases.

Submucosal Injection Using Epinephrine-Added Saline in Cold Snare Polypectomy for Colorectal Polyps Shortens Time Required for Resection: A Randomized Controlled Study.

Aims: Immediate bleeding after cold snare polypectomy (CSP) for colorectal polyps might interfere with confirmation of residuals and prolong the time required for resection. We investigated whether submucosal epinephrine-added saline injection reduces the time required for the CSP procedure.

Methods: We conducted a single-center, prospective, randomized controlled trial (clinical trial registration number: UMIN000046770).

Associations of time-dependent changes in phosphorus levels with cardiovascular diseases in patients undergoing hemodialysis: results from the Japan Dialysis Active Vitamin D (J-DAVID) randomized clinical trial.

Background: While the risk of exceeding the standard range of phosphorus levels has been investigated, the impact of the degree of fluctuations has not been investigated.

Methods: Data were derived from the Japan Dialysis Active Vitamin D trial, a 4-year prospective, randomized study involving 976 patients without secondary hyperparathyroidism undergoing hemodialysis in Japan. Laboratory data were collected every 6 months and the primary outcome was the time to the occurrence of cardiovascular events.

Cystic Kidney Diseases That Require a Differential Diagnosis from Autosomal Dominant Polycystic Kidney Disease (ADPKD).

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary cystic kidney disease, with patients often having a positive family history that is characterized by a similar phenotype. However, in atypical cases, particularly those in which family history is unclear, a differential diagnosis between ADPKD and other cystic kidney diseases is important. When diagnosing ADPKD, cystic kidney diseases that can easily be excluded using clinical information include: multiple simple renal cysts, acquired cystic kidney disease (ACKD), multilocular renal cyst/multilocular cystic nephroma/polycystic nephroma, multicystic kidney/multicystic dysplastic kidney (MCDK), and unilateral renal cystic disease (URCD).

Effects of alfacalcidol on cardiovascular outcomes according to alkaline phosphatase levels in the J-DAVID trial.

In the Japan Dialysis Active Vitamin D (J-DAVID) trial, oral alfacalcidol numerically, but not significantly, increased the risk of cardiovascular events among patients undergoing hemodialysis. Because the cardiovascular effect of alfacalcidol could be modulated by bone turnover status, this post-hoc analysis of the J-DAVID examined how alkaline phosphatase (ALP), a more precise marker of bone turnover than parathyroid hormone (PTH), modifies the impact of alfacalcidol. The J-DAVID was a 48-month, open-label, randomized controlled trial comparing oral alfacalcidol with no vitamin D receptor activators use in terms of cardiovascular events among 976 hemodialysis patients without secondary hyperparathyroidism.

Dialysate calcium, alfacalcidol, and clinical outcomes: A post-hoc analysis of the J-DAVID trial.

The selection of dialysate calcium concentration (D-Ca) is still controversial among chronic hemodialysis (HD) regimens. We examined the trajectories of CKD MBD parameters among the J-DAVID trial participants to see the effect of D-Ca and alfacalcidol. The trial was an open-label randomized clinical trial including 976 HD patients with intact PTH of 180 pg/mL or lower which compared the users of vitamin D receptor activator (oral alfacalcidol) and non-users over a median of 4 years.

Small intestinal bleeding and stricture caused by Meckel's diverticulum.

A 44-year-old man presented to our hospital with lower gastrointestinal bleeding. We performed balloon-assisted enteroscopy, which revealed diverticulum and stricture at the ileum. The patient underwent segmental small bowel resection and diagnosed with Meckel's diverticulum.

Serum phosphate as an independent factor associated with cholesterol metabolism in patients undergoing hemodialysis: a cross-sectional analysis of the DREAM cohort.

Background: Hyperphosphatemia is a risk factor for cardiovascular outcomes in patients with chronic kidney disease. In an experimental model, hyperphosphatemia promoted atherosclerosis by activating sterol regulatory element-binding protein 2, which controls cholesterol homeostasis. In the present study, we hypothesized that serum phosphate level is associated with cholesterol metabolism in patients with kidney failure.